Izmirly Peter M, Saxena Amit, Sahl Sara K, Shah Ummara, Friedman Deborah M, Kim Mimi Y, Buyon Jill P
Division of Rheumatology, Department of Medicine, New York University School of Medicine, New York, New York, USA.
Division of Rheumatology, Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York, New York, USA.
Ann Rheum Dis. 2016 Jun;75(6):1161-5. doi: 10.1136/annrheumdis-2015-208311. Epub 2015 Dec 1.
Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit.
In this retrospective study of cases enrolled in the Research Registry for NL, inclusion was restricted to anti-SSA/Ro-exposed cases presenting with isolated advanced heart block in utero who either received fluorinated steroids within 1 week of detection (N=71) or no treatment (N=85). Outcomes evaluated were: development of endocardial fibroelastosis, dilated cardiomyopathy and/or hydrops fetalis; mortality and pacemaker implantation.
In Cox proportional hazards regression analyses, fluorinated steroids did not significantly prevent development of disease beyond the AV node (adjusted HR=0.90; 95% CI 0.43 to 1.85; p=0.77), reduce mortality (HR=1.63; 95% CI 0.43 to 6.14; p=0.47) or forestall/prevent pacemaker implantation (HR=0.87; 95% CI 0.57 to 1.33; p=0.53). No risk factors for development of disease beyond the AV node were identified.
These data do not provide evidence to support the use of fluorinated steroids to prevent disease progression or death in cases presenting with isolated heart block.
疾病超出房室(AV)结与心脏新生儿狼疮(NL)死亡率增加相关。曾考虑使用氟化类固醇治疗孤立性心脏传导阻滞以预防疾病进展,但关于疗效的已发表数据有限且不一致。本研究评估给予氟化类固醇治疗孤立性高度传导阻滞是否能预防疾病超出AV结发展并带来生存获益。
在这项针对NL研究登记处登记病例的回顾性研究中,纳入标准仅限于子宫内出现孤立性高度心脏传导阻滞且在检测后1周内接受氟化类固醇治疗(N = 71)或未接受治疗(N = 85)的抗SSA/Ro抗体暴露病例。评估的结局包括:心内膜弹力纤维增生症、扩张型心肌病和/或胎儿水肿的发生;死亡率和起搏器植入情况。
在Cox比例风险回归分析中,氟化类固醇并不能显著预防疾病超出AV结发展(校正风险比[HR]=0.90;95%置信区间[CI]为0.43至1.85;p = 0.77),降低死亡率(HR = 1.63;95% CI为0.43至6.14;p = 0.47)或避免/预防起搏器植入(HR = 0.87;95% CI为0.57至1.33;p = 0.53)。未发现疾病超出AV结发展的危险因素。
这些数据不支持在出现孤立性心脏传导阻滞的病例中使用氟化类固醇预防疾病进展或死亡。